NRAS gene mutations occur in less than 5% of appendiceal cancers, being present in some cases of low- and high-grade appendiceal mucinous neoplasms, in which missense mutation C>A is identified in codon 181, with amino acid replacement Gln61Lys [29], in mucinous adenocarcinomas of the appendix [23] and appendiceal goblet cell adenocarcinomas [26,37]. Here, NRAS is linked to goblet cell carcinoma.